Hypertrophic Cardopmyopathy Flashcards
Definition of HOCM
-Unexplained increased wall thickness >15mm in the LV wall.
Prevalence of HOCM
1:500
Very common
Histological features of HOCM
- Disarray of cell-to-cell arrangements
- Fibrosis
- Disorganization of cellar architecture
CHF symptoms in HOCM are due to…?
- Diastolic dysfunction
- LV outflow obstruction (dynamic)
About 5-10% of HOCM progressed to CHFrEF
Why to HOCM patients have chest pain
-Supply and demand mismatch due to thickened vessels and small vessel disease from increased collagen deposition in the intima and media
Yamaguchi HCM
Apical HCM
- Japan 25% of all cases
- Giant T wave inversions in the precordial leads
Echocardiographic findings in Apical HCM
- Localized hypertrophy in the distal ventricle beyond the chordae
- Wall thickness of at least 15mm or a ratio of apical to basal >1.5
How do we screen for HCM
First degree relatives every 12-18 months with EKG and Echo
Starting at age 12
When do we stop screening for HCM
Continue into middle age as their can be late-onset phenotypic expression
What risk does a mutant HCM gene impart?
> 95% lifetime risk of developing the clinical and/or phenotypic evidence of disease
What is the typical LHC findings in apical HCM?
Spade like configuration of the LV cavity at the end diastole
Apical end-systolic LV cavity obliteration
What are the risk markers for primary prevention for HCM
- Prolonged or repetitive episodes of NSVT
- LV thickness >30mm
- FHx of SCD in 1st degree relative
- Failure to increase BP >20mmHg with exercise
- Unexplained syncope
What are the primary medications for pAF and HCM to maintain NSR?
- Amiodarone
- Disopyramide
What is the procedure of choice for patients with symptomatic HCM?
- After medical therapy:
- Surgical myomectomy
When should asymptomatic young patients be considered for non-pharmalogical treatment of HCM?
-WIth gradients >75mmHg
